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Adhesion G protein-coupled receptor V1
Symbols ADGRV1 ; FEB4; GPR98; MASS1; USH2B; USH2C; VLGR1; VLGR1b
External IDs OMIM602851 MGI1274784 HomoloGene19815 IUPHAR: GeneCards: ADGRV1 Gene
RNA expression pattern
PBB GE GPR98 gnf1h01727 s at tn.png
PBB GE GPR98 gnf1h07403 at tn.png
PBB GE GPR98 gnf1h07404 at tn.png
More reference expression data
Species Human Mouse
Entrez 84059 110789
Ensembl ENSG00000164199 ENSMUSG00000069170
UniProt Q8WXG9 Q8VHN7
RefSeq (mRNA) NM_032119 NM_054053
RefSeq (protein) NP_115495 NP_473394
Location (UCSC) Chr 5:
90.53 – 91.16 Mb
Chr 13:
81.1 – 81.63 Mb
PubMed search [1] [2]

G protein-coupled receptor 98, also known as GPR98 or VLGR1, is a protein that in humans is encoded by the GPR98 gene.[1] Several alternatively spliced transcripts have been described.[1]

The adhesion GPCR Very Large GPCR receptor 1 (Vlg1R1) is the largest GPCR known, with a size of 6300 amino acids and consisting of 90 exons.[2] There are 8 splice variants of VlgR1, named VlgR1a-1e and Mass1.1-1.3. The N-terminus consists of 5800 amino acids containing 35 Calx-beta domains, one pentraxin domain, and one epilepsy associated repeat. Mutations of VlgR1 have been shown to result in Usher's syndrome. Knockouts of Vlgr1 in mice have been shown to phenocopy Usher's syndrome and lead to audigoenic seizures.


This gene encodes a member of the adhesion-GPCR family of receptors.[3] The protein binds calcium and is expressed in the central nervous system. It is also known as very large G-protein coupled receptor 1 because it is 6300 residues long. It contains a C-terminal 7-transmembrane receptor domain, whereas the large N-terminal segment (5900 residues) includes 35 calcium binding Calx-beta domains, and 6 EAR domains.


The Sea Urchin genome has a homolog of VLGR1 in it.[4]

Clinical significance[edit]

Mutations in this gene are associated with Usher syndrome 2 and familial febrile seizures.[1]


  1. ^ a b c "Entrez Gene: GPR98 G protein-coupled receptor 98". 
  2. ^ Sun, JP; Li, R; Ren, HZ; Xu, AT; Yu, X; Xu, ZG (May 2013). "The very large g protein coupled receptor (vlgr1) in hair cells". J Mol Neurosci 50 (1): 204–14. doi:10.1007/s12031-012-9911-5. 
  3. ^ Stacey M, Yona S (2011). AdhesionGPCRs: Structure to Function (Advances in Experimental Medicine and Biology). Berlin: Springer. ISBN 1-4419-7912-3. 
  4. ^ Whittakera, Charles A.; Bergerone, Karl-Frederik; Whittlec, James; Bruce, P. (2006). "Brandhorste, Robert D. Burked, Richard O. Hynes. The echinoderm adhesome". Developmental Biology 300 (1): 252–266. doi:10.1016/j.ydbio.2006.07.044. 

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